C1 Inhibitor and Diagnosis of Hereditary Angioedema in Newborns
نویسندگان
چکیده
منابع مشابه
Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor
Until recently it was assumed that hereditary angioedema is a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity and protein in plasma were described. Since then numerous patients and families with that condition have been reported. Most of the patients by far were women. In many of the affec...
متن کاملSelf-administration of intravenous C1 esterase inhibitor in hereditary angioedema.
somal dominant disorder that results in episodes of acute edema in various organs, including the gastrointestinal tract, skin and larynx. It is estimated to affect about 1 in 50 000–100 000 people. The symptoms, including abdominal pain, laryngeal edema and subcutaneous edema, usually begin in childhood and persist throughout life with unpredictable severity. Hereditary angioedema is classified...
متن کاملHereditary angioedema without deficiency of C1 inhibitor: response to therapy
Results Nineteen patients have been identified (2M:17F; 20-60 years old). The following therapies were oriented: combined contraceptive substitution for progestagen (10/19); treatment with progestagen (2/19); tranexamic acid (15/19): 1250mg (2), 1000mg (1), 750 (5), 500 mg (4), 250 mg (1); oxandrolon (5/19) (0.5 mg-5mg/day), danazol 200mg/day (1/19) and combined therapy woth oxandrolon and tran...
متن کاملC1-inhibitor concentrate for treatment of hereditary angioedema.
BACKGROUND Hereditary angioedema due to C1 inhibitor deficiency is characterized by recurrent acute attacks of swelling that can be painful and sometimes life-threatening. METHODS We conducted two randomized trials to evaluate nanofiltered C1 inhibitor concentrate in the management of hereditary angioedema. The first study compared nanofiltered C1 inhibitor concentrate with placebo for treatm...
متن کاملTreatment of hereditary angioedema with plasma-derived C1 inhibitor
BACKGROUND Plasma-derived C1 inhibitor (C1-INH) concentrate is a treatment option for acute hereditary angioedema (HAE) attacks and is considered the standard-of-care in many countries, although it is not yet available in the United States. Studies are still being conducted to establish its safety and efficacy as required by the FDA. OBJECTIVE To review the medical literature to determine if ...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1994
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199402000-00012